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A 7-month-old boy was referred to our department with a 2-week history characterized by weight loss. Initial physical examination was unremarkable, except for hepatosplenomegaly. Laboratory tests revealed increased liver enzymes (alanine amino transferase: 98 U/L, normal value 10–44; aspartate aminotransferase: 105 U/L, normal value 10–34 with normal-range bilirubin: 0.19 mg/dL, normal value 0–1); severe anemia (7.7 g/dL, normal value 9.5–13.5) with neutrophilic leukocytosis (white blood cell: 19,410 × 103/μL, normal value 6000–13,000; neutrophils 9900 × 103/μL, normal value 1500–8500), and elevated inflammatory markers (erythrocyte sedimentation rate: 120 mm/hour, normal value 2–20; C-reactive protein: 6.41 mg/dL, normal value 0–0.5). A hepatic ultrasonography revealed a large number of hyperechoic hepatic lesions. A computerized axial tomography scan confirmed the presence of hepatic lesions consistent with liver abscesses (Fig. 1). Chronic granulomatous disease (CGD) was suspected. The positivity of superoxide anion production and nitroblue tetrazolium tests confirmed our diagnosis. A liver biopsy showed the presence of a necrotizing granulomatous hepatitis; blood and biopsy cultures did not detect fungi or bacterial agents. As reported in the literature, the search for microorganisms during infections in patients with CGD, despite proper procedure, is often negative (1). Staphylococcal species and fungi (eg, candida, aspergillus) are the most common organisms isolated in liver abscesses in CGD; in particular, Staphylococcus aureus accounts for more than 50% of liver infections (2). Therefore, empirical treatment with antibiotics (clindamycin: 30 mg · kg−1 · 24 h−1 IV and ciprofloxacin: 12.5 mg · kg−1 · 24 h−1 IV) and antifungal medications (voriconazole: 15 mg · kg−1 · 24 h−1 IV) was started and maintained for 40 days. CGD is an inherited primary immunodeficiency disease, occurring with a frequency of 1 in 200,000 (3).
There is no definite consensus about the management of liver abscesses in CGD. Most authors emphasize the importance of a surgical intervention (4,5). In our case, as reported by other authors (6), the decision not to drain the abscesses was based on the dramatic response to antibiotics that occurred in 1 month (Fig. 2); surgery would have been considered an option if no result arose after 2 months of antibiotic treatment.
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. Clinical features, long-term follow-up and outcome of a large cohort of patients with chronic granulomatous disease: an Italian multicenter study. Clin Immunol 2008; 126:155–164.
2. Guide SV, Stock F, Gill VL, et al
. Reinfection, rather than persistent infection, in patients with chronic granulomatous disease. J Infect Dis 2003; 187:845–853.
3. Seger RA. Modern management of chronic granulomatous disease. Br J Haematol 2008; 140:255–266.
4. Garcia-Eulate R, Hussain N, Heller T, et al
. CT and MRI of hepatic abscess in patients with chronic granulomatous disease. Am J Roentgenol 2006; 187:482–490.
5. Chen LE, Minkes RK, Shackelford PG, et al
. Cut it out: managing hepatic abscesses in patients with chronic granulomatous disease. J Pediatr Surg 2003; 38:709–713.
6. Fehon R, Mehr S, La Hei E, et al
. Two-year-old boy with cervical and liver abscesses. J Paediatr Child Health 2008; 44:670–672.